Postgraduate Medical Journal (May 1976) 52, 310-312.

Rhabdomyosarcoma of the heart

M. RAMUM.B.B.S., Ph.D.

Government Medical Laboratory, 33A North Street, Kingston, Jamaica

SummaryA case of rhabdomyosarcoma of the heart is described,which is one of the rare primary tumours of the heartdocumented in the literature. The presenting post-mortem appearances were noted.

IntroductionAlthough a rhabdomyosarcoma (Anderson, 1971)

is among the common malignant tumours of the softparts of the body, it is a rare primary tumour of theheart.

Case reportAn 81-year-old male was brought to the Casualty

Department, Kingston Public Hospital, Jamaica,and was pronounced dead on arrival. According tothe relative, the main complaint of the deceased hadbeen, for some time, recurrent abdominal pain andloss of weight.

External examination revealed the deceased tobe moderately built and emaciated. There was noclubbing of the fingers or evidence of external injuryor violence. There was marked cyanosis.

Internal examination showed the lungs to becongested and oedematous and there were signs ofchronic bronchitis with emphysema. The pericardiumand mediastinal glands were normal.The heart weighed 300 g and the valves were

healthy. The coronary arteries were patent butshowed arteriosclerotic changes. Marked arterio-sclerotic changes were also seen in all the majorblood-vessels. There was a bulging mass, yellowishwhite in colour, in the upper end of the anterior wallof the left ventricle (nearer to the mitral valve). Itwas a non-polypoid mass measuring 2 x 1 5 x 1 cmand growing from the myocardium and partiallyfilling the left ventricular cavity. The base of thetumour involved the anterior wall of the left ven-tricle. Microscopically, HE-stained sections showeda fairly cellular tumour extensively infiltrating theheart muscle. The cells were arranged diffusely insome areas and in others were separated by connec-tive tissue septa giving an alveolar pattern. Thetumour cells irregularly lined the 'alveoli' with somecells lying free in the lumina (Fig. 1). The cells wereround with abundant eosinophilic cytoplasm and

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FIG. 1. HE-stained section of the tumour, showing extensive infiltration of the heartmuscle.

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Case reports 311

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FIG. 2. HE-stained section showing multinucleated cells and some strap cells.

of

FIG. 3. PTAH-stained section showing some striated cells.

some cells had vacuolated cytoplasm. Multinucleatedgiant cells could be seen. Strap cells were seen insome areas (Fig. 2). There were also a few mitoticareas. PTAH-stained sections revealed some striatedcells (Fig. 3).

Discussion and conclusionPrichard (1951) was of the opinion that the

status of this tumour had not yet been established.Straus and Merliss (1945) reviewed the worldliterature on primary tumours of the heart and

concluded that the incidence of this condition was019 %. Gould (1968), mentioning the variationin the incidence of primary cardiac tumours, listedvarious types of which only seven cases wereprimary tumours of the heart and only one caseof rhabdomyosarcoma. The most recent review,which is the one for the years 1951-1973 by Hardinet al. (1974) was of seventeen patients with pri-mary cardiac tumours, of which one was a caseof rhabdomyosarcoma.As a rule, primary tumours of the heart are very

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312 Case reports

rare and this is especially so in the case of rhabdo-myosarcoma of the heart. Clinical diagnosis of thiscondition has not been recorded, and diagnosisbefore death would depend on the condition causingsymptoms brought about by interference with cardiacmechanism. The tumour described in this report wasa silent one.

AcknowledgmentI wish to thank Dr D. C. Watler, Director, Government

Medical Laboratory for his encouragement to write thispaper and the Chief Medical Officer, Ministry of Health, forallowing me to publish this paper.

ReferencesANDERSON, W.A.D. (1971) Pathology, 6th Edn, pp. 578-687.Mosby Co., U.S.A.

GOULD, S.E. (1968) Pathology of Heart and Blood-lVessels,3rd Edn, p. 876. Charles C. Thomas, Springfield, Illinois.

HARDIN, N.J.H., WILSON III, J.M., GRAY, G.F. & GAY,W.A., JR (1974) Experience with primary tumors of theheart. Clinical and pathological study of 17 cases. JohnsHopkins Medical Journal, 134, 141.

PRICHARD, R. (1951) Tumors of the heart. Review of thesubject and report of 150 cases. Archives of Pathology,51, 98.

STRAUS, R. & MERLISS, R. (1945) Primary tumor of heart.Archives of Pathology, 39, 74.

Postgraduate Medical Journal (May 1976) 52, 312-315.

Aplasia of the right lung and calcifying epithelioma in associationwith Goldenhar's syndrome

M. M. KENAWI* J. A. S. DICKSONM.Ch., F.R.C.S.Ed., F.R.C.S. F.R.C.S. Ed.,F.R.C.S.

* The Hospitalfor Sick Children, Great Ormond Street, London WCJN 3JH, andDepartment of Paediatric Surgery, 30 Guilford Street, London WCIN JEH

SummaryA case of Goldenhar's syndrome (oculoauriculoverte-bral dysplasia) with the rare association of aplasia ofone lung is presented with a discussion of the clinicalfindings and the aetiology. The major abnormalities,pulmonary, renal and the undergrowth of the jaw wereall right-sided, confirming previous reports. This childdeveloped a calcifying epithelioma and this is cited assupport for the theory of the origin of these lesionsfrom epidermoid cysts.

IntroductionGoldenhar's syndrome (1952) or oculoauriculo-

vertebral dysplasia is a complex syndrome withocular, auricular, oral and musculoskeletal features(Magalini, 1971). Cardiac anomalies occur occasion-ally but aplasia of the lung has been reported onlyonce before (Gorlin et al., 1963). Although epibulbardermoid cysts are frequent, this is the first reportedassociation with a calcifying epithelioma of Mal-herbe.

* Present address: Cardiothoracic Surgery Registrar,The London Chest Hospital, Bonner Road, LondonE2 9JX.

Case reportM.M. presented to the Hospital for Sick Children,

Great Ormond Street, London, at the age of3 months with dyspnoea, recurrent cough and poorfeeding, and failure to thrive. Her weight was only3-4 kg (below 3rd centile). The heart sounds werebest heard in the right chest, with a pansystolicmurmur at the right sternal margin and a loud pul-monary second sound. The right hemithorax wasunder-developed and immobile. There were peduncu-lated accessory auricles along the line from thetragus to the angle of the mouth on both sides. Con-genital heart disease with dextro-cardia due to rightpulmonary atelectasis was diagnosed.A chest radiograph showed displacement of the

mediastinum to the right and no aeration of the rightlung (Fig. 1). Abdominal radiographs were normal.An ECG showed left ventricular hypertrophywith marked axis rotation. Cardiac catheteriza-tion, via the inferior vena cava, revealed pul-monary hypertension and a patent ductus arteriosuswith bi-directional shunting. Angiocardiographydemonstrated a single left pulmonary artery con-tinuous with the parent trunk. The right pulmonaryartery was atretic (Fig. 2). A small sequestrated right

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